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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunodeficiency with thymoma (Good syndrome, GS) is a rare, adult-onset condition that is characterized by thymoma, hypogammaglobulinemia, and low numbers of peripheral B cells. CD4+ T lymphopenia and an inverted CD4:CD8+ T-cell ratio may be present. Here we report 5 patients with GS and infectious complications who were seen at 3 institutions between 1983 and 1999. Three patients had recurrent sinopulmonary infections, 3 had severe cytomegalovirus (CMV) disease, and 1 had Pneumocystis carinii pneumonia. Review of the literature identified 46 other reports of infections in GS patients. The infections reported in all 51 patients included recurrent sinopulmonary infection (19 cases with documented respiratory pathogens), generally with encapsulated bacteria, most often Haemophilus influenzae (11 cases); CMV disease (5 cases); bacteremia (7 cases); oral or esophageal candidiasis (6 cases); persistent mucocutaneous candidiasis (5 cases); chronic diarrhea (5 cases with documented stool pathogens); urinary tract infections (4 cases); P. carinii pneumonia (3 cases); tuberculosis (2 cases); Kaposi sarcoma (1 case); disseminated varicella (1 case); candidemia (1 case); wound infection with Clostridium perfringens (1 case); Mycoplasma arthritis (1 case); and other infections. Patients with GS present with a spectrum of sinopulmonary infections and pathogens similar to common variable immunodeficiency (CVID). Compared with patients with CVID, opportunistic infections, including severe CMV disease, P. carinii pneumonia, and mucocutaneous candidiasis, appear to be more common in patients with GS, and patients with GS may have a worse prognosis. GS should be ruled out in patients with thymoma or CVID who develop severe, especially opportunistic, infections. Treatment with intravenous immune globulin is recommended for all patients with GS.
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PMID:Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. 1130 88

A 63-year-old man was admitted to our hospital for treatment of hypogammaglobulinemia with thymoma (Good's syndrome). Tests for immunological function showed an abnormality in humoral immunity with decreases in the proportion of cells bearing B-cell markers in the peripheral blood and bone marrow. The patient was found to have Campylobacter fetus sepsis caused by the hypogammaglobulinemia due to humoral immunodeficiency, and he was given gamma-globulin supplement. Thymectomy was performed due to enlargement of the thymoma after 4 years of follow-up and the pathological diagnosis was thymoma of the non-encapsulated, epithelial spindle cell type. Although there was no recurrence of thymoma, the hypogammaglobulinemia remained unchanged and the patient continued to suffer from repeated infections. Thus, we describe the case of a patient with Good's syndrome associated with independent humoral immunodeficiency in whom the effect of thymectomy for hypogammaglobulinemia was negative. In this respect, thymectomy was only beneficial when the thymoma proliferated and seemed to be more threatening than the hypogammaglobulinemia for the patient.
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PMID:Thymoma associated with hypogammaglobulinemia (Good's syndrome): report of a case. 1199 14

In this paper, clinical data of 49 adult patients with agammaglobulinaemia (syn. hypogammaglobulinaemia), 15 cases of X-linked agammaglobulinaemia (XLA) and 34 of common variable immunodeficiency (CVID) are reviewed. Although immunoglobulin substitution largely abolished life-threatening respiratory tract infections, considerable infectious and non-infectious morbidity was still encountered in these patients. Almost all patients suffered from chronic or recurrent upper and lower airway infections, mainly caused by Haemophilus influenzae and pneumococci. The lower respiratory tract infections led to cumulative damage to the respiratory tract, especially in XLA patients. Also the incidence of infections outside the respiratory tract (giardiasis, Campylobacter jejuni infections) was more common in XLA patients than in CVID patients. Nodular lymphoid hyperplasia was only found in CVID. A variety of other non-infectious complications were seen especially in CVID. Neoplastic complications occurred in nine patients (two cases of thymoma, two colorectal cancer, one gastric carcinoma, two haematological malignancies, two cases of skin cancer). Six patients died (five XLA patients and one CVID patient, from infectious and non-infectious causes).
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PMID:Hypogammaglobulinaemia: cumulative experience in 49 patients in a tertiary care institution. 1216 71

Oral candidiasis is associated with defects in cell-mediated immunity and is common among patients undergoing cytotoxic chemotherapy, or corticosteroid or antibiotic therapy, and those patients seropositive for AIDS and HIV (human immunodeficiency virus). This paper demonstrates the important role of cell-mediated immunity in oral candidiasis in 2 cases of thymoma associated with myasthenia gravis. Both suffered from recurrent oral candidiasis after a thymectomy, radiotherapy, and chemotherapy. There was an initial good response to conventional antifungal therapy, which later became refractory. Lymphocyte subset quantitation showed a T cell deficiency and a decreased CD4/CD8 ratio. Levamisole, an immunomodulator, or an immunopotentiating drug was added as adjunctive therapy in combination with oral nystatin treatment. Oral candidiasis responded favorably, and substantial relief was obtained with a concurrent increase in T cells and the CD4/CD8 ratio. These findings clearly demonstrate a significant role of cell-mediated immunity in oral candidiasis, and that eradication of infection is dependent on the host defense mechanism.
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PMID:Levamisole aids in treatment of refractory oral candidiasis in two patients with thymoma associated with myasthenia gravis: report of two cases. 1247 22

A subgroup of thymoma patients is affected by severe immunodeficiency clinically resembling an HIV infection (Good syndrome). These individuals are characterized by B lymphopenia with B-lymphopoiesis deficiency. To investigate the pathogenesis of this unique condition, we studied the T-cell repertoire in blood and bone marrow samples by heterogeneity length analysis of CDR3 beta variable regions of the T-cell receptor (spectratyping). While no alterations were found in the peripheral blood, we detected an oligoclonal population of beta variable 8 (BV8) CD8(+) T cells in 5 of 5 bone marrow samples. No lymphocyte expansions were found in the bone marrow of 2 thymoma patients with normal B-cell counts, 2 healthy donors, and 3 patients with diseases unrelated to thymoma. These data suggest that an immune response toward an unknown antigen is taking place in the bone marrow of B-lymphopenic thymoma patients. We propose that BV8 CD8(+) T cells may play a role in the pathogenesis of this immunodeficiency syndrome.
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PMID:Clonal expansion of CD8+ BV8 T lymphocytes in bone marrow characterizes thymoma-associated B lymphopenia. 1251 21

Three male patients, aged 56, 40 and 52 years, presented with recurrent infections and clinically suspected immunodeficiency, which was confirmed by the presence of hypogammaglobulinaemia. In one patient, no obvious underlying disease was identifiable, and primary immunodeficiency syndromes were considered in the differential diagnosis. A thorough diagnostic work-up revealed the presence of non-Hodgkin lymphoma in an iliac crest biopsy specimen. The second patient was found to have a thymoma. In the third patient, the immunodeficiency was the key to the ultimate diagnosis of multiple myeloma, which was still asymptomatic at that stage. Establishing the underlying condition had important therapeutic and prognostic consequences in these patients.
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PMID:[Immunodeficiency in adult patients as the first sign of a primary underlying condition]. 1257 66

Parathymic syndromes are systemic disorders that occur in association with thymoma. One such parathymic syndrome, hypogammaglobulinemia, was initially identified by Good in 1954 and has been referred to as Good syndrome. Patients with this syndrome develop a variety of recurrent infections due to the associated immunodeficiency. We describe a patient with cytomegalovirus encephalitis associated with Good syndrome and discuss the pathologic findings present on autopsy. The possibility of a cytomegalovirus infection should be considered early in the evaluation of patients with Good syndrome if appropriate clinical symptoms are present.
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PMID:Fatal subacute cytomegalovirus encephalitis associated with hypogammaglobulinemia and thymoma. 1258 34

Good's syndrome is an infrequent disorder associated with humoral immunodeficiency and thymoma. Patients usually present severe or chronic infections. The most frequent are sinopulmonary infections sometimes associated with bronchiectasias. Another complication is chronic diarrhea, frequently associated with malabsorption, which can be due to a mucosal lesion, infection with gastrointestinal pathogens, or bacterial overgrowth. We describe a patient with Good's syndrome and chronic diarrhea due to bacterial overgrowth.
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PMID:[Good's syndrome and chronic diarrhea]. 1268 Nov 17

Thymic tumors represent a unique neoplastic disease associated with various immune-mediated syndromes. Immune impairment is generically recognized to be associated with thymoma. Hypogammaglobulinemia and recurrent pulmonary infections in thymoma patients define Good's syndrome. Apart from sporadic reports focusing on this topic, there is still a lack of knowledge on immune assessment and clinical sequelae in thymoma patients. The present study was performed to evaluate immunoglobulin levels, CD19(+) B lymphocytes, and CD3(+) T lymphocytes in a large series of thymoma patients from a single institution. The occurrence of recurrent severe infections was related to immunological findings to identify the possible correlation with the immunodeficiency status. Eighteen patients (eight males, ten females, mean age: 56 years, range: 19-75) with a pathological diagnosis of thymic tumor were studied. Six patients suffered from clinical recurrent pulmonary infections. Blood samples were collected to measure serum immunoglobulins and analyze immunophenotype. Low T lymphocyte number was found in 22% of the patients. T lymphocytosis was present in one patient. Panhypogammaglobulinemia was found in 4 of 18 patients (22%). Conversely B lymphopenia was a frequent finding in this series of thymoma patients (9 of 18, 50%). Five of six patients (83%) with recurrent infections had B lymphopenia, while only two (33%) had panhypogammaglobulinemia. B lymphopenia often occurred in this series of thymoma patients and was related to susceptibility to recurrent infections more than hypogammaglobulinemia. Therefore, immunophenotype has to be monitored in follow-up of thymoma patients because it may reveal significant abnormalities.
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PMID:B-cell lymphopenia and hypogammaglobulinemia in thymoma patients. 1271 6

Thymic T cell development is characterized by sequential selection processes to ensure generation of a self-tolerant, immuncompetent mature T cell repertoire. Malfunction of any of these selection processes may potentially result in either immunodeficiency or autoimmunity. Myasthenia gravis (MG) is a typical autoimmune manifestation of thymic epithelial tumors (thymomas) and is related to the capacity of these tumors to generate and export mature T cells. Analysis of the factors that lead to autoimmunization in thymomas will help to understand the mechanisms that prevent MG under physiological conditions in humans. In a comparison of MG(+) and MG(-) thymomas, we could show that only thymomas capable of generating mature CD45RA+CD4+ T cells are associated with MG (p < 0.0001), while terminal thymopoiesis was abrogated in MG(-) thymomas. In particular, acquisition of the CD27+CD45RA+ phenotype appears to be a critical checkpoint of late T cell development in the human thymus and may play an important role in the prevention of autoimmunity. Moreover, MG(-) thymomas were virtually depleted of regulatory (CD4+CD25+) T cells (regT), while regT were readily detectable in MG(+) thymomas, albeit at significantly reduced numbers compared to control thymuses. Thus, in MG(+) thymoma patients, thymectomy apparently also results in removal of a regulatory T cell pool and may explain the frequent temporary postoperative deterioration of MG in these patients.
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PMID:Pathomechanisms of paraneoplastic myasthenia gravis. 1457 52


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